Everything about Mullerian Agenesis totally explained
Mullerian agenesis refers to a condition in a female where the
mullerian ducts fail to develop and a uterus won't be present. It is the second most common cause of Primary
amenorrhea.
The condition is also called
MRKH or
Mayer-Rokitansky-Küster-Hauser Syndrome, named after
August Franz Joseph Karl Mayer,
Carl Freiherr von Rokitansky,
Hermann Küster, and
G.A.Hauser.
Signs and symptoms
A woman with this condition is hormonally normal, that's she'll enter puberty with development of secondary sexual characteristics including
thelarche and
adrenarche. Her chromosome constellation will be 46,XX. Ovulation usually occurs. Typically, the vagina is shortened and intercourse will be difficult and painful. Medical examination supported by
gynecologic ultrasonography demonstrates a complete or partial absence of the
cervix,
uterus, and
vagina.
Since there's no uterus, women with MRKH can't carry a pregnancy. However, it's possible for these women to have genetic offspring by
in vitro fertilisation (IVF) and surrogacy. Uterine transplantation is currently not a treatment that can be offered as the technology is still in its infancy.
Women with MRKH typically discover the condition when, during puberty years, the menstrual cycle doesn't start. Some women find out earlier through surgeries for other conditions, such as a hernia.
Other, less common symptoms include kidney problems, hearing loss, and bone malformations.
Prevalence
The estimated prevalence is 1 in 5000 women. A genetic cause is likely (see
OMIM
).
Treatment
Although there are treatments to increase the comfort in sexual intercourse, there are none to let the woman herself become pregnant. There are plans by UK and Swedish doctors for a uterine transplant that would allow these women to carry their own child, but no transplant has yet been successful in producing a child. Since the women do have ovaries, women with this condition can have genetic children through
IVF with
embryo transfer to a
gestational carrier. Some women also choose to adopt.
It may be necessary to use vaginal dilators or surgery to develop a functioning vagina to allow for satisfactory sexual intercourse. A number of surgical approaches have been used. In the McIndoe procedure a skin graft is applied to form an artificial vagina. After the surgery, dilators are still necessary to prevent vaginal
stenosis. The Vecchietti procedure is a
laparoscopic procedure that has been shown to result in a vagina that's comparable to a normal vagina in patients with Mullerian agenesis.
Emotional help is available in various support groups across the internet.
Further Information
Get more info on 'Mullerian Agenesis'.
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